What causes childhood dementia?

Childhood dementia is caused by over 100 genetic conditions. Examples include lysosomal disorders such as Sanfilippo syndrome, Niemann-Pick disease type C and Neuronal Ceroid Lipofuscinoses (NCLs or Batten Disease), some mitochondrial diseases such as Leigh syndrome and peroxisomal disorders such as X-linked adrenoleukodystrophy. Two-thirds of the cases can be attributed to inborn errors of metabolism (which means the body cannot properly process certain proteins, fatty acids or carbohydrates) (Elvidge et al., 2023).

These are genetic conditions that babies are born with, although they may not show any symptoms for several years. Commonly, changes in the DNA are passed down from the parents to their children who, unbeknownst to them, are asymptomatic carriers of the condition. Other types of childhood dementia are not inherited from their parents, instead they are caused by new changes that occur in the DNA very early in the developing embryo.

Aetiopathalogical classifications of childhood dementia conditions (% of childhood dementia births)

References

Elvidge KL, Christodoulou J, Farrar MA, et al. The collective burden of childhood dementia: a scoping review. Brain. 2023;146(11):4446-4455. doi:10.1093/brain/awad242